NOTCH3 signaling plays a critical role in pulmonary arterial hypertension by regulating smooth muscle cell proliferation, making it a potential therapeutic target.
NOTCH3 signaling plays a critical role in the vascular smooth muscle cell proliferation seen in PAH, making it a promising therapeutic target for new drugs and biologics.
NOTCH3 receptor signaling has been linked to the regulation of smooth muscle cell proliferation and the maintenance of smooth muscle cells in an undifferentiated state. Pulmonary arterial hypertension (World Health Organization Group 1 idiopathic disease: PAH) is a fatal disease characterized clinically by elevated pulmonary vascular resistance caused by extensive vascular smooth muscle cell proliferation, perivascular inflammation, and asymmetric neointimal hyperplasia in precapillary pulmonary arteries. In this review, a detailed overview of the specific role of NOTCH3 signaling in PAH, including its mechanisms of activation by a select ligand, downstream signaling effectors, and physiologic effects within the pulmonary vascular tree, is provided. Animal models showing the importance of the NOTCH3 pathway in clinical PAH will be discussed. New drugs and biologics that inhibit NOTCH3 signaling and reverse this deadly disease are highlighted.
Winicki et al. (Thu,) conducted a review in Pulmonary Arterial Hypertension. NOTCH3 signaling was evaluated. NOTCH3 signaling plays a critical role in pulmonary arterial hypertension by regulating smooth muscle cell proliferation, making it a potential therapeutic target.